II. Epidemiology

  1. Incidence: Up to 39 cases per 100,000
  2. Usual onset at 20 to 40 years
  3. Gender preponderance in men by 2:1 ratio
  4. Ethnicity
    1. Most commonly affects white patients
    2. Rarely affects black or asian patients

III. Pathophysiology

  1. Idiopathic condition
  2. Usually associated with Gluten Sensitive Enteropathy

IV. Symptoms: Precede lesion onset by 8 to 12 hours

  1. Intense Pruritus
  2. Skin burning

V. Signs

  1. Grouping of lesions may occur (herpetiform-like)
  2. Altered pigmentation at sites of healed lesions
  3. Polymorphous collection of lesions
    1. Urticarial wheals
    2. Vesicles
    3. Bullae
    4. Erythematous Papules
  4. Symmetric distribution of lesions on limbs and trunk
    1. Common sites
      1. Elbows and knees
      2. Buttocks and Shoulders
      3. Sacral area
    2. Uncommon sites
      1. Scalp and hairline
      2. Face and posterior neck
    3. Rare sites
      1. Palms and soles
      2. Mucus Membranes

VI. Differential Diagnosis

  1. Pemphigus hermetiformis

VII. Labs

  1. Complete Blood Count
    1. Eosinophilia
  2. Histology
    1. Dermal Papillae with Neutrophil microabscesses
    2. Dermal inflammatory infiltrate
    3. Subepidermal vessicles
    4. Blisters in the lamina lucida
    5. Lymphohistiocytic infiltrate at dermal vessels
  3. Immunofluorescence
    1. Granular IgA deposits in Dermal Papillae tips
  4. Other autoimmune lab associations variably present
    1. Antinuclear Antibody
    2. Antithyroid Microsomal Antibody

VIII. Complications

  1. Gluten Sensitive Enteropathy associated conditions
    1. Steatorrhea
    2. Abnormal D-Xylose Absorption
    3. Anemia
    4. Atrophic Gastritis
    5. Achlorhydria
    6. Gastrointestinal Lymphoma
  2. Autoimmune Conditions
    1. Thyroid Disease
    2. Type I Diabetes Mellitus
    3. Systemic Lupus Erythematosus
    4. Vitiligo
    5. Sjogren's Syndrome

IX. Management

  1. Medications
    1. First-Line: Dapsone
    2. Alternative: Sulfapyradine
  2. Dietary Management
    1. Gluten-Free Diet (improvement within 6-12 months)
    2. Elemental Diet (improvement may be seen in weeks)

X. Monitoring

  1. See Dapsone (requires G6PD and monitoring of CBC)

XI. Course

  1. Prolonged course over years
  2. Spontaneous remission in one third of patients

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Ontology: Dermatitis Herpetiformis (C0011608)

Definition (NCI) A chronic autoimmune skin disorder characterized by the development of pruritic papulovesicular and bullous lesions in the elbows, knees, buttocks, and back. It is associated with an increased expression of HLA-A1, HLA-B8, and HLA-DR3 antibodies.
Definition (MSH) Rare, chronic, papulo-vesicular disease characterized by an intensely pruritic eruption consisting of various combinations of symmetrical, erythematous, papular, vesicular, or bullous lesions. The disease is strongly associated with the presence of HLA-B8 and HLA-DR3 antigens. A variety of different autoantibodies has been detected in small numbers in patients with dermatitis herpetiformis.
Concepts Disease or Syndrome (T047)
MSH D003874
ICD9 694.0
ICD10 L13.0
SnomedCT 200899006, 156354007, 111196000
English Dermatitis Herpetiformis, Duhring Disease, Duhring's Disease, Duhrings Disease, Disease, Duhring's, Disease, Duhring, DERMATITIS HERPETIFORMIS, DUHRING DIS, DUHRINGS DIS, dermatitis herpetiformis, dermatitis herpetiformis (diagnosis), Dermatitis Herpetiformis [Disease/Finding], disease duhring's, duhrings disease, herpetiformis dermatitis, Dermatitis;herpetiformis, duhring disease, duhring's disease, Dermatitis herpetiformis, Duhring's disease, Dermatosis herpetiformis, DH - Dermatitis herpetiformis, Duhring-Brocq disease, Dermatitis herpetiformis (disorder), dermatitis; herpetiformis, dermatosis; herpetiformis, herpetiformis; dermatitis, herpetiformis; dermatosis, Duhring, Dermatitis herpetiformis [dup] (disorder)
French DERMATITE HERPETIFORME, Dermatite herpétiforme de Dühring, Dermatite herpétiforme, Maladie de Duhring-Brocq
Portuguese DERMATITE HERPETIFORME, Dermatite herpetiforme, Dermatite Herpetiforme, Doença de Duhring
Swedish Dermatitis herpetiformis
Japanese ホウシンジョウヒフエン, 皮膚炎-ヘルペス状, Duhring病, ジューリング疱疹状皮膚炎, デューリング疱疹状皮膚炎, デューリング病, ヘルペス状皮膚炎, 疱疹状皮膚炎, 皮膚炎-疱疹状
Czech dermatitis herpetiformis, Herpetiformní dermatitida
Finnish Ihokeliakia
German DERMATITIS HERPETIFORMIS, Dermatitis herpetiformis [Duhring], Dermatitis herpetiformis, Dermatitis herpetiformis Duhring, Duhring-Krankheit
Italian Morbo di Duhring, Dermatite erpetiforme
Korean 포진상 피부염
Polish Choroba Duhringa, Zapalenie skóry opryszczkowate
Hungarian Dermatitis herpetiformis
Norwegian Dermatitis herpetiformis, Duhrings sykdom
Spanish dermatitis herpetiforme [dup] (trastorno), enfermedad de Duhring-Brocq, dermatitis herpetiforme (trastorno), dermatitis herpetiforme, dermatosis herpetiforme, enfermedad de Duhring, Dermatitis herpetiforme, Dermatitis Herpetiforme, Enfermedad de Duhring
Dutch dermatitis; herpetiformis, dermatose; herpetiform, herpetiformis; dermatitis, herpetiformis; dermatose, dermatitis herpetiformis, Dermatitis herpetiformis, Duhring, ziekte van, Ziekte van Duhring